Christopher R. Dermarkarian, Veeral Shah, Richard C. Allen
Congenital orbital fibrosis (COF) is a nonprogressive, unilateral, congenital process in which variable fibrosis is demonstrated in the orbit, resulting in restrictive strabismus, upper eyelid malposition, and axial displacement of the globe. We present 4 new pediatric cases of COF and discuss factors that impact visual development. We also describe a patient with local compressive optic neuropathy/edema who underwent optic nerve sheath fenestration (ONSF) for visual preservation.
Literature review and retrospective case series.
Four male COF patients (mean age of 11 months) were examined. Two patients presented with decreased ocular motility of the affected eye. Two patients presented with exophthalmos, and one presented with enophthalmos. Two patients presented with ptosis, and one presented with eyelid retraction. Two patients presented with optic nerve atrophy, and one presented with optic nerve edema. Magnetic resonance imaging demonstrated involvement of the superior, medial, and inferior rectus and superior oblique muscles in 3 patients and the lateral rectus and inferior oblique muscles in 2 patients. Three patients underwent orbitotomy. Histology was consistent with fibrosis. Three patients demonstrated amblyopia, and 2 responded to treatment. The patient with optic nerve edema underwent ONSF. At 4 months’ follow-up, the edema had resolved.
COF can present with either anterior or posterior globe displacement. Patients must undergo a complete ophthalmic evaluation to identify modifiable factors. Strabismus and ptosis should be addressed for optimal visual development. Amblyopia therapy should be instituted quickly. Patients who present with active optic nerve edema may benefit from ONSF for local compressive optic neuropathy.
Leonardo Lando, Marina O S Paiva, Luciano S Pereira
A 56-year-old man underwent a shunting procedure in childhood due to hydrocephalus secondary to meningitis. Along the following years, he manifested recurrent tearing, foreign body sensation, and slow “eye shrinking”. External clinical exam confirmed enophthalmos, poor lid apposition to the globe, and inferior ectropion bilaterally [Figure 1]a. Biomicroscopy showed dry eye syndrome bilaterally. Computed tomography scans of the brain and orbits revealed ventriculomegaly (asterisks), ventriculoperitoneal shunt valves (one implant at arrow), posterior displacement of eyeballs, and significant orbital remodeling [Figure 1]b. The patient was diagnosed with silent brain syndrome, a rare form of progressive enophthalmos after intracranial pressure resolution.,
Liza M. Cohen, Larissa A. Habib & Michael K. Yoon
To investigate via volumetric analysis whether orbital fat atrophy occurs in late post-traumatic enophthalmos.
An IRB-approved retrospective cohort study identified patients with diagnoses of both orbital fracture and enophthalmos with a CT orbits >3 months after injury. Exclusion criteria were surgical repair, other orbital disease or surgery, adjacent sinus disease, and an abnormal contralateral orbit. Images were analyzed using OsiriX imaging software (v.9.0.2, Pixmeo, Switzerland). Total orbital volume and orbital fat volume for the fractured and normal contralateral orbits were measured via three-dimensional volume rendering assisted region-of-interest computation. Enophthalmos was measured radiographically. Paired samples t-tests were used to compare orbital fat and total orbital volumes between the fractured and normal contralateral orbits.
Thirteen patients met the inclusion criteria. The numbers of patients with each fracture pattern were floor (4), medial wall (4), floor/medial wall (3), zygomaticomaxillary complex (floor+lateral wall) (1), zygomaticomaxillary complex+medial (inferior/medial/lateral walls) (1). Mean time from injury to CT scan was 21.8 ± 16.3 months. Comparing the fractured and normal contralateral orbits, there was a statistically significant decrease in orbital fat volume (mean difference 0.9 ml (14.2%), p = .0002) and increase in total orbital volume (mean difference 2.0 ml (7.0%), p = .0001). One ml orbital volume change was responsible for 0.83 mm enophthalmos.
In addition to an increase in total orbital volume, orbital fat loss occurs with late post-traumatic enophthalmos due to unrepaired fractures. This suggests correction of bony change alone may be insufficient in some cases, and the use of custom implants may compensate for fat atrophy.
Serena Fragiotta , Massimiliano Sepe , Andrea Perdicchi , Luca Scuderi , Maria Trani & Gianluca Scuderi
A 62-year-old white woman presented with a diagnosis of blue rubber bleb nevus syndrome (BRBNS). The right eye appeared enophthalmic, yet the patient complained of episodes of right proptosis on bending forward. The remainder of the examination was unremarkable. Orbital ultrasound (US) in an upright posture revealed a single low reflectivity cavity (4.27 mm x 2.82 mm) of uncertain interpretation. In a forward-leaning posture the lesion increased in size (maximum thickness of 13.72 mm), demonstrating multiple low reflectivity spaces with highly reflective septae. This case first reports the use of US with postural changes to assess the presence of orbital venous malformation in BRBNS. The expansile nature upon postural changes supports the venous origin of the orbital lesion.
Vibha Baldev, Shailja Tibrewal
A 24-year-old man with a deviation of the right eye noted since childhood presented with significant enophthalmos, limitation of elevation, and globe retraction on attempted elevation. A disproportionately small right hypotropia was suggestive of a restrictive pathology, and forced-duction testing confirmed tightness of the inferior rectus muscle. Clinical features resembled those of orbital floor fracture with inferior rectus entrapment. Computed tomography disclosed no fracture but did reveal an abnormal structure arising from the orbit adjacent to the inferior rectus origin and attaching to the globe close to the optic nerve in the inferolateral aspect.
Yongrong Ji, Yixiong Zhou, Qin Shen, Wei Xu, Shengfang Ge, Lixu Gu, Xianqun Fan
To establish a linear measuring method in computed tomographic (CT) images to predict the displacement of the globe late after orbital blowout fracture.
Subjects were retrospectively included. Inclusion criteria were as follows: (1) adult subjects (≥18 years old at the time of trauma); (2) unilateral orbital medial‐wall and/or floor fractures; (3) CT examination at least 30 days after trauma. Exclusion criteria were as follows: (1) facial or orbital fracture extending to other parts of the orbit than medial‐wall and/or floor; (2) history of orbital or ocular abnormality other than the orbital trauma; (3) severe ocular trauma accompanied by the orbital trauma; (4) orbital fracture treated surgically before the CT examination. A co‐ordinate system was built based on the orbital CT scans. Displacements of orbital walls, displacement of the globe and relative location of the fracture site were measured. Correlations between the variables were investigated.
Ninety‐nine per cent of fracture sites of the medial wall and 100% of fracture sites of the floor were posterior to the centre of the unaffected globe. The affected globe moved significantly medially (p < 0.001) and backwards (p < 0.001) in pure medial‐wall fracture; backwards (p < 0.001) and downwards (p = 0.017) in pure floor fracture; and medially (p < 0.001), backwards (p < 0.001) and downwards (p < 0.001) in medial‐wall and floor fractures. Displacement of the globe was correlated with displacements of the orbital walls, and the regression formulae were therefore fitted. Application of the formulae revealed that the same extent of orbital wall displacement caused more displacement of the globe in female patients than in male patients.
A linear measuring method in a three‐dimensional co‐ordinate system was established to identify the displacements of orbital walls and the displacement of the globe in orbital blowout fractures. The regression formulae generated in this study might be used in clinical practice to predict late displacement of the globe by measuring the displacements of orbital walls.
Young Woong Mo, Sung Woo Kim, Hea Kyeong Shin
Enophthalmos is one of the most distressing complications of orbital fracture, and when faced with a blowout fracture, plastic surgeons often find it difficult to determine the need for surgical correction. Although a number of studies have been conducted on this topic, no study has yet been performed using a set of measurable parameters.
We quantitatively measured orbital fracture areas (OFA), volumes (OFV), and medial rectus muscle cross-sectional ratios in patients with an isolated medial orbital wall fracture defect during a 5-year period from 2014 to 2018. Only conservatively treated patients constituted the study cohort. Enophthalmos was measured by two plastic surgeons at ≥6 months after trauma. We analyzed correlations between various parameters and degrees of late enophthalmos in the study cohort.
Significant correlations were observed between several parameters and late enophthalmos. Multiple regression analysis resulted in the following coefficients: -0.449 is constant (p = 0.017), 0.596 and 0.460 for OFA (p = 0.000) and OFV (p = 0.005), respectively. However, no significant relationship was observed between enophthalmos and medial rectus muscle cross-sectional ratios (p = 0.340).
The results of this study enable the degree of late enophthalmos in conservatively treated patients to be predicted using OFV and OFA values. We suggest five indications that require surgical correction without long-term clinical follow-ups: (1) OFA ≥ 1.90 cm2, (2) OFV ≥ 1.00 cm3, (3) 1.30 OFA + OFV > 5.32, (4) signs of muscle incarceration, and (5) signs of severe retrobulbar hematoma. The more satisfactory the five indications are, the greater is the need for surgery.
Urfalioglu, Selma; Acipayam, Can; Güler, Mete; Sahin, Bedia; Kütükcü, Meliha Kübra
Neuroblastoma is the most common type of extracranial solid tumor during childhood. Clinical presentation includes ipsilateral ptosis, myosis, anhydrosis and enophthalmos. The case of a 2.5-year-old boy who had a complaint of constriction of the left pupil for 3 days is presented. In the physical examination, the pupil of the OD was moderately dilated; there was myosis on the OS and ptosis on the left eyelid. Horner syndrome was considered due to these findings. History of the patient revealed that a central venous catheter insertion procedure was tried from the left side.
Alexandra Manta, Robert A. Goldberg
Pembrolizumab is a highly selective immune checkpoint inhibitor that targets the programmed cell death protein (PD-1) receptor on lymphocytes and has been approved for the treatment of malignant melanoma. A 59-year-old woman had been treated for 1 year with pembrolizumab for melanoma metastatic to lung, spleen, and central nervous system. She noted changes of her facial appearance with temporal wasting and sunken eyes while denying any decrease in body weight. Exophthalmometry was 11 mm for the right eye (OD) and 10 mm for the left eye (OS). (Fig A, B) Axial magnetic resonance imaging (MRI) scans performed 6 months apart (Fig C, D) demonstrated a significant change in globe position with 4.7 mm recession on the right and 4.1 mm on the left. There were no signs of orbital metastasis or inflammation. She had no other medical or iatrogenic factors to explain loss of orbital volume and enophthalmos. (Magnified version of Fig A–D is available online at www.aaojournal.org).
Yoshiaki Sakamoto, Yoshitake Yamada, Minoru Yamada, Yoichi Yokoyama, Kazuo Kishi, Masahiro Jinzaki
Enophthalmos is defined as a backward and downward displacement of the globe into the bony orbit. Clinically, more than 2 mm of enophthalmos is noticeable and a 5% increase in the total volume of the orbital cavity is sufficient to result in significant enophthalmos.1